First off, I want to announce that the winner of the drawing for the book White Picket Fences from last week’s entries was…Stacey Cartlidge! I am sending you a private message about how to get your book! Thank you to everyone who commented to win. And now, on to our regularly scheduled programing.
At the Sparks’ house, November is for purple. Purple pumpkins, door wreaths, awareness bracelets, clothes and Converse. It’s the color of Epilepsy Awareness and we don it all in support of my daughter Shelby. That’s her on the 50 ft billboard at the local Children’s hospital this month. As Shelby has grown, I have honestly gotten lazier about educating others and spreading awareness. But my best friend’s daughter woke me up from my slumber by deciding to write a research paper on Dravet Syndrome (the epilepsy syndrome my Shelby was born with) for her Freshman Comp class at Auburn. She wanted to interview me and I was thrilled and honored – especially when I saw her intelligent and well-thought-out questions!
As I responded to Avery’s inquiries in writing, I realized that a large portion of my bloggy friends have never heard this part of our story or know much about seizures. So with Avery’s permission, I am publishing her questions and my answers here:
Sparks, Lauren. Personal Interview, 9 November 2018.
- Can you start by providing a brief explanation of your situation? (relation, family members + dynamic, introduce Shelby, her current state) When was Shelby diagnosed; what was the process? Anything else you’d like to add or think needs to be added?
Shelby, my daughter is the only member of our family ever diagnosed with Dravet Syndrome. She began having seizures at 4 months of age, but was not diagnosed until 8 years of age because it is considered rare, but was even more so then. It was not even a doctor that suggested she be tested for it, it was me (after speaking with someone else with a Dravet child and then researching it online). At the time, the blood test for diagnosis had a 6 month waiting list for results so it was quite a process. Even though the prognosis for Dravet is dim, it felt like a relief to finally have answers for her. By that time she had already been on 2 medications that are contraindicated for her condition that made her seizures worse. She developed on the slow side of normal until about 18 months of age and then had an abrupt halt in global development. She is now 18 and is intellectually, socially and emotionally about 2 years of age. Her seizures remain uncontrolled after many years of treatment and upwards of 20 different medications. She is currently enrolled in a drug trial that is not going as well as we had hoped to control her episodes.
- In the sources I have read, there is a general consensus that before the first birthday, the first seizure will occur, after the first birthday, the style and longevity of seizures shift and mental deterioration occurs, and when entering early adolescence, the frequency of seizures decreases. They also agree that mental stabilization occurs around age six as well as increased behavioral issues.
- Are these things true in Shelby’s case? Can and will you provide examples of why or why not?
Yes, with one exception. Shelby’s first couple of seizures were localized to one limb, but by not quite 5 months of age she had her first tonic clonic seizure. She continues to have tonic clonic seizures as well as myoclonic seizures. Over the years she has also had absence and atypical absence seizures. The frequency, length and duration of them have varied greatly over the years. And for the last several, her tonic clonic are primarily nocturnal. She has also experienced clusters and status epilepticus. For the most part, her seizures did improve in the teenage years. In the toddler years, she had to be sedated every time she had a tonic clonic seizure to get it to stop. Now they typically only last 1-2 minutes and stop on their own. Even so, she had a cluster seizure about a year ago that lasted 14 hours – even after being taken by ambulance to the hospital and having medical intervention. Her myoclonic seizures, though not as scary or dangerous, can still last several hours and interfere with functioning. We are blessed that Shelby has never really had behavioral issues. She is very sweet natured, even tempered and loving.
- One woman wrote about different strategies families she interviewed shared with her including: using a stroller, planning for different situations in advance, having emergency protocol that can be shared with the hospitals in the area, and different coping mechanisms like self-care and taking time to breathe.
- What strategies have you created over the years for Shelby’s well-being, your personal care, as well as your family?
Shelby has outgrown strollers, but we do have a wheelchair we can use when we need to. She is perfectly capable of walking but is slow and tires easily, so we plan ahead and have it with us if we will need to walk long distances (like the mall, amusement park or airport). My husband and I were not smart enough when Shelby was younger to have an emergency protocol in place and, thankfully, emergencies are so rare now. Our biggest self-care “indulgence” is taking care of our marriage. The statistics on divorce in special needs families have been reported at times as high as 85%. Not only do my husband and I value our relationship, but know how difficult taking care of Shelby would be alone. We keep a “staff” of 3 or more attendants at all times who are trained to take care of Shelby so that we can have regular date nights and time alone.
- Different researchers mentioned the effects having a sibling with Dravet Syndrome can have. They explained different strategies parents use to incorporate the sibling as well as help them cope and understand.
- How has Allie’s life been affected?
She has always been aware that Shelby is different and it’s made her more compassionate of people that are different – especially special needs individuals. She has even thought, at times, that she might want to be a special ed teacher. Growing up, there were always things we couldn’t do because of Shelby’s limitations, but we tried to work around whatever we could to make Allie’s childhood as normal as possible. I think, as a whole, she would say she really hasn’t missed much.
- What did you do as parents to help Allie understand and respond positively?
When she was young we tried to minimize the danger and severity of Shelby’s seizures. She has been afraid at times, but for the most part doesn’t internalize it. When she was about 8 years old, I took her to a counselor to make sure she was handling it all as well as she appeared and the therapist agreed that Allie was very well adjusted. We have always told her that if her feelings about things ever changed she could talk to us.
- Multiple families mentioned social isolation that increased as their child got older.
- What has been your experience, if any, with that type of isolation?
- How were you affected and how have you responded?
- How have your relationships with others outside of your immediate family, such as extended family and friends, changed?
- How has your relationship with your husband changed?
- What have you done to keep your relationships stable and healthy?
We definitely experienced this. When you are a young mom, your friendships revolve around playmates for your children. As Shelby aged and her cognition fell further and further behind, it was hard for us to make and keep couple friends because Shelby couldn’t play with other children in a meaningful way. We were involved in life groups with families of similar aged kids and struggled to contribute a lot to the conversations because our life with her was so different. Grades and sports and extracurriculars didn’t exist in the same way for her. Even now, as most of our friends have teens and are very busy with all of their activities, loneliness is a very real issue for us. Thankfully, my husband and I really enjoy each other’s company, but it can’t completely replace the joy of close friendships.
- What would you personally consider the most challenging aspect of your situation?
In temperament and personality, Shelby is a joy, but her daily care is labor intensive. She still wears adult sized pull-ups and needs complete assistance to toilet. She also cannot assist in her own bathing or dressing. She can’t be left alone so she needs constant supervision. She also won’t sleep alone in her own room so she has a twin bed at the foot of the master bed. It’s hard to have, for all practical purposes, an adult sized toddler.
- How did you respond to Shelby’s first seizure?
- How did you respond to Shelby’s seizures shortly after her diagnosis?
- How has your response changed?
- How has it stayed the same?
I honestly don’t remember a whole lot about her very first one. I have probably blocked it out. I do remember that we drove her to the hospital ourselves instead of calling an ambulance. And because she was tonic we wouldn’t have been able to buckle her into a car seat so I held her in the front seat of the car while my husband drove. Not the smartest. Every seizure she had her first couple of years scared me more than I can explain. She had a single seizure that lasted an hour and 15 minutes in which she was given so much valium at the hospital that she stopped breathing and had to be intubated. She was only about 18 months old. As she has aged and the severity of her seizures has decreased, they are not as frightening to me, but still hard to watch her suffer.
- What was your biggest worry/fear when Shelby was diagnosed?
- How has that changed or stayed the same?
My biggest worry/fear was that she would never learn and never be independent. That fear as been realized and we have found that although it’s hard, it is not the end of the world. Now my biggest fear is still her future, but it’s about finding an appropriate spot for her when we no longer feel it is the right thing to do for us to care for her in our home. And we spend a lot of time planning for her care when my husband and I are no longer living. SUDEP (Sudden Unexplained Death in Epilepsy) is also a very real fear.
- Different researchers mentioned the lack of research being performed for Dravet Syndrome.
- In your experience, is a lack of research obvious?
- How do you respond?
I think it is obvious that there is not nearly enough research being done on Dravet. The first indication? When you mention it to most people, the response is “what?” They have never heard of it. We have been very involved over the years with the Epilepsy Foundation and the Dravet Syndrome Foundation. Both have granted research funds. We even raised money ourselves to fund a research grant in Shelby’s name. When given the opportunity, we also participate in research ourselves.
- Is there anything you think I should have asked or anything you would like to add that you want people to learn or understand?
The next big need for Shelby is good, competent adult neurology care. Providers in the adult sector who know anything about Dravet Syndrome are few and far between. Not to mention that the number of providers who will take her Medicaid might be even lower. When Shelby turns 26 and can no longer be carried on our insurance, there are no other options for her as she will never be able to work. This is a big issue in the special needs community at large and must be addressed for continuity of care for complex medical patients like Shelby.
That was a trip down memory lane that I don’t often take, but important for me to look back on all that God has seen our family through. Dravet Syndrome is only 1 of many conditions or circumstances that can cause a seizure. In fact, 1 in 6 people will experience one in their life time. So my challenge for you, for Epilepsy Awareness Month is this –eftx.org to find out. It could save a life!